Skip to main content

Advertisement

SpringerLink
Log in

Neuromyelitis Optica-AQP4: An Update

  • VASCULITIS (LUIS R. ESPINOZA, SECTION EDITOR)
  • Published:
Current Rheumatology Reports Aims and scope Submit manuscript

Abstract

Devic disease (neuromyelitis optica [NMO]) is an idiopathic inflammatory demyelinating and necrotizing disease characterized by optic neuritis and transverse myelitis, either simultaneously or in isolation. NMO is often idiopathic but may also be associated with systemic autoimmune disease. The prognosis of NMO is severe, especially in those with early and recurrent relapses. MRI studies have revealed that most frequently, there is a long spinal cord lesion that extends through three or more vertebral segments in length. NMO-IgG is the first antibody marker for any inflammatory central nervous system disorder and is both sensitive and specific for NMO. The identification of NMO-IgG in patients with recurrent optic neuritis or longitudinally extensive myelitis and its ability to predict subsequent relapse support the concept of a spectrum of NMO disorders. Treatment in the acute phase includes intravenous steroids and plasma exchange therapy. Immunosuppressive agents are recommended for the prophylaxis of relapses.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Allbutt TC. On the ophthalmoscopic signs of spinal disease. Lancet. 1870;1:76–8.

    Article  Google Scholar 

  2. Devic E. Myélite subaiguë compliquée de névrite optique. Bull Med (Paris). 1894;8:1033–4.

    Google Scholar 

  3. Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG. The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology. 1999;53:1107–14.

    PubMed  CAS  Google Scholar 

  4. Wingerchuk DM, Lennon VA, Pittock SJ, Lucchinetti CF, Weinshenker BG. Revised diagnostic criteria for neuromyelitis optica. Neurology. 2006;66:1485–9.

    Article  PubMed  CAS  Google Scholar 

  5. Lennon VA, Wingerchuk DM, Kryzer TJ, Pittock SJ, Lucchinetti CF, Fujihara K, et al. A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis. Lancet. 2004;364(9451):2106–12.

    Article  PubMed  CAS  Google Scholar 

  6. Seze J, Lebrun C, Stojkovic T, et al. Is Devic’s neuromyelitis optica a separate disease? A comparative study with multiple sclerosis. Mult Scler. 2003;9:521–5.

    Article  PubMed  Google Scholar 

  7. Harisdangkul V, Doorenbos D, Subramony SH. Lupus transverse myelopathy: better outcome with early recognition and aggressive high-dose intravenous corticosteroid pulse treatment. J Neurol. 1995;242:326–31.

    Article  PubMed  CAS  Google Scholar 

  8. Bergamaschi R, Ghezzi A. Devic’s neuromyelitis optica: clinical features and prognostic factors. Neurol Sci. 2004;25:S364–7.

    Article  PubMed  Google Scholar 

  9. Cabre P, Heinzlef O, Merle H, et al. MS and neuromyelitis optica in Martinique (French West Indies). Neurology. 2001;56:507–14.

    PubMed  CAS  Google Scholar 

  10. Merle H, Olindo S, Bonnan M, et al. Natural history of the visual impairment of relapsing neuromyelitis optica. Ophthalmology. 2007;114(4):810–5. Epub 2006 Nov 30.

    Article  PubMed  Google Scholar 

  11. Papais-Alvarenga RM, Miranda-Santos CM, Puccioni-Sohler M. Optic neuromyelitis syndrome in Brazilian patients. J Neurol Neurosurg Psychiatry. 2002;73(4):429–35.

    Article  PubMed  CAS  Google Scholar 

  12. Balser BH. Neuromyelitis optica. Brain. 1936;59:353–65.

    Article  Google Scholar 

  13. Erb W. Über das Zusammenkommen von Neuritis optica und Myelitis subacute. Arch Psychiatr Nervenkr 1879–1980;1:146–57.

    Google Scholar 

  14. Beck GM. A case of diffuse myelitis associated with optic neuritis. Brain. 1927;50:687–703.

    Article  Google Scholar 

  15. Weinshenker BG. Neuromyelitis optica: what it is and what it might be. Lancet. 2003;361:889–90.

    Article  PubMed  Google Scholar 

  16. Field HB. A case of neuomyelitis optica. Ill Med J. 1961;119:362–7.

    PubMed  CAS  Google Scholar 

  17. Pittock SJ, Lennon VA, Krecke K, et al. Brain abnormalities in neuromyelitis optica. Arch Neurol. 2006;63:390–6.

    Article  PubMed  Google Scholar 

  18. Keefe RJ. Neuromyelitis optica with increased intracranial pressure. Arch Ophthal. 1957;57:110–1.

    CAS  Google Scholar 

  19. O’Riordan JI, Gallagher HL, Thompson AJ, et al. Clinical, CSF, and MRI findings in Devic’s neuromyelitis optica. J Neurol Neurosurg Psychiatry. 1996;60:382–7.

    Article  PubMed  Google Scholar 

  20. • Jarius S, Franciotta D, Paul F, et al. Cerebrospinal fluid antibodies to aquaporin-4 in neuromyelitis optica and related disorders: frequency, origin, and diagnostic relevance. J Neuroinflammation 2010;7:52. In the cohort analyzed in this study, testing for CSF AQP4 antibodies did not improve the sensitivity and specificity of the current diagnostic criteria for NMO. The substantial lack of intrathecal AQP4 antibody synthesis in patients with NMOSD may reflect the unique localization of the target antigen at the blood–brain barrier and is important for our understanding of the immunopathogenesis of the disease.

    Article  PubMed  Google Scholar 

  21. Tashiro K, Ito K, Maruo Y, et al. MR imaging of spinal cord in Devic disease. J Comput Assist Tomogr. 1987;11:516–7.

    Article  PubMed  CAS  Google Scholar 

  22. de Seze J, Stojkovic G, Breteau G, et al. Acute myelopathies. Clinical, laboratory and outcome profiles in 79 cases. Brain. 2001;124:1509–21.

    Article  PubMed  Google Scholar 

  23. Cree B, Goodin D, Hauser S. Neuromyelitis optica. Semin Neurol. 2002;22(2):105–22.

    Article  PubMed  Google Scholar 

  24. Cabrera-Gomez J, Quevedo-Sotolongo, Gonzalez-Quevedo A, et al. Brain magnetic resonance imaging findings neuromyelitis optica. Mult Scler. 2007;13:186–92.

    Article  PubMed  Google Scholar 

  25. Pittock SJ, Weinshenker BG, Lucchinetti CF, Wingerchuk DM, Corboy JR, Lennon VA. Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression. Arch Neurol. 2006;63(7):964–8.

    Article  PubMed  Google Scholar 

  26. Barkhof F, Scheltens P, Valk J, Waalewijn C, Uitdehaag BM, Polman CH. Serial quantitative MR assessment of optic neuritis in a case of neuromyelitis optica, using gadolinium-“enhanced” STIR imaging. Neuroradiology. 1991;33:70–1.

    Article  PubMed  CAS  Google Scholar 

  27. Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR. IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med. 2005;202(4):473–7. Epub 2005 Aug 8.

    Article  PubMed  CAS  Google Scholar 

  28. Amiry-Moghaddam M, Ottersen OP. The molecular basis of water transport in the brain. Nat Rev Neurosci. 2003;4(12):991–1001.

    Article  PubMed  CAS  Google Scholar 

  29. Kurtzke JF, Park CS, Oh SJ. Multiple sclerosis in Korea. Clinical features and prevalence. J Neurol Sci. 1968;6(3):463–81.

    Article  PubMed  CAS  Google Scholar 

  30. Pittock S, Lennon V, de Seze, et al. Neuromyelitis optica and non-organ-specific autoimmunity. Arch Neurol. 2008;65(1):78–83.

    Article  PubMed  Google Scholar 

  31. Harada T, Ohashi T, Harada C, et al. A case of bilateral optic neuropathy and recurrent transverse myelopathy associated with perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA). J Neuroophthalmol. 1997;17:254–6.

    Article  PubMed  CAS  Google Scholar 

  32. Mochizuki A, Hayashi A, Hisahara S, Shoji S. Steroid-responsive Devic’s variant in Sjögren’s syndrome. Neurology. 2000;54(6):1391–2.

    PubMed  CAS  Google Scholar 

  33. • Paira S, Benegas M, Ortiz A. Association of neuromyelitis optic (NMO) with autoimmune disorders: report of two cases and review of the literature. Clin Rheumatol 2010;29:1335–8. In this article, the authors present two patients with NMO (Devic disease), one associated with SLE and the other with Hashimoto’s thyroiditis. They emphasize the importance of ruling out other entities throughout the follow-up. NMO is seldom described in patients with lupus, which may be due to the rare occurrence of this association, sometimes leading to its misdiagnosis.

    Article  Google Scholar 

  34. Lucchinetti CF, Mandler RN, McGavern D, et al. A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica. Brain. 2002;125:1450–61.

    Article  PubMed  Google Scholar 

  35. Stansbury FC. Neuromyelitis optica (Devic’s disease): presentation of five cases with pathologic study, and review of the literature. Arch Ophthalmol. 1949;42:292–335.

    CAS  Google Scholar 

  36. Weinshenker BG, O’Brien PC, Petterson TM, et al. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol. 1999;46(6):878–86.

    Article  PubMed  CAS  Google Scholar 

  37. Cree BA, Lamb S, Morgan K, Chen A, Waubant E, Genain C. An open label study of the effects of rituximab in neuromyelitis optica. Neurology. 2005;64(7):1270–2.

    Article  PubMed  CAS  Google Scholar 

  38. Roemer SF, Parisi JE, Lennon VA, et al. Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. Brain. 2007;130(Pt 5):1194–205. Epub 2007 Feb 4.

    Article  PubMed  Google Scholar 

  39. Misu T, Fujihara K, Nakamura M, et al. Loss of aquaporin-4 in active perivascular lesions in neuromyelitis optica: a case report. Tohoku J Exp Med. 2006;209(3):269–75.

    Article  PubMed  Google Scholar 

  40. Matiello M, Jacob A, Wingerchuk DM, Weinshenker BG. Neuromyelitis optica. Curr Opin Neurol. 2007;20(3):255–60.

    Article  PubMed  Google Scholar 

  41. Tesar JT, McMillan V, Molina R, Armstrong J. Optic neuropathy and central nervous system disease associated with primary Sjögren’s syndrome. Am J Med. 1992;92(6):686–92.

    Article  PubMed  CAS  Google Scholar 

  42. Williamson PM. Neuromyelitis optica following infectious mononucleosis. Proc Aust Assoc Neurol. 1975;12:153–5.

    PubMed  CAS  Google Scholar 

  43. Doutlik S, Sblova O, Kryl R, Novak M. Neuromyelitis optica as a parainfectious complication of varicella. Cesk Neurol Neurochir. 1975;38:238–42.

    PubMed  CAS  Google Scholar 

  44. Chusid MJ, Williamson SJ, Murphy JV, Ramey LS. Neuromyelitis optica (Devic disease) following varicella infection. J Pediatr. 1979;95:737–8.

    Article  PubMed  CAS  Google Scholar 

  45. al-Deeb SM, Yaqub BA, Khoja WO. Devic’s neuromyelitis optica and varicella. J Neurol. 1993;240:450–1.

    PubMed  CAS  Google Scholar 

  46. Blanche P, Diaz E, Gombert B, Sicard D, Rivoal O, Brezin A. Devic’s neuromyelitis optica and HIV-1 infection. J Neurol Neurosurg Psychiatry. 2000;68:795–6.

    Article  PubMed  CAS  Google Scholar 

  47. Barbizet J, Degos JD, Meyrignac C. Acute neuromyelitis optica and acute pulmonary tuberculosis (author’s trans.). Rev Neurol (Paris). 1980;136:303–9.

    CAS  Google Scholar 

  48. Hughes RA, Mair WG. Acute necrotic myelopathy with pulmonary tuberculosis. Brain. 1977;100:223–38.

    Article  PubMed  CAS  Google Scholar 

  49. Silber MH, Willcox PA, Bowen RM, Unger A. Neuromyelitis optica (Devic’s syndrome) and pulmonary tuberculosis. Neurology. 1990;40:934–8.

    PubMed  CAS  Google Scholar 

  50. Vernant JC, Cabre P, Smadja D, et al. Recurrent optic neuromyelitis with endocrinopathies: a new syndrome. Neurology. 1997;48(1):58–64.

    PubMed  CAS  Google Scholar 

  51. Fardet L, Généreau T, Mikaeloff Y, Fontaine B, Seilhean D, Cabane J. Devic’s neuromyelitis optica: study of nine cases. Acta Neurol Scand. 2003;108(3):193–200.

    Article  PubMed  CAS  Google Scholar 

  52. Poppe AY, Lapierre Y, Melançon D, et al. Neuromyelitis optica with hypothalamic involvement. Mult Scler. 2005;11(5):617–21.

    Article  PubMed  Google Scholar 

  53. Berman M, Feldman S, Alter M, Zilber N, Kahana E. Acute transverse myelitis: incidence and etiologic considerations. Neurology. 1981;31(8):966–71.

    PubMed  CAS  Google Scholar 

  54. •• Espinosa G, Mendizábal A, Mínguez S, et al. Transverse myelitis affecting more than 4 spinal segments associated with systemic lupus erythematosus: clinical, immunological, and radiological characteristics of 22 patients. Semin Arthritis Rheum. 2010;39(4):246–56. Epub 2008 Nov 20. In this study, the main clinical and immunologic features of 22 patients, including 2 previously unreported cases, were compared with a group of 32 SLE patients with transverse myelitis affecting less than 4 spinal segments collected from 3 reported series.

    Article  PubMed  Google Scholar 

  55. Lavalle C, Pizarro S, Drenkard C, Sánchez-Guerrero J, Alarcón-Segovia D. Transverse myelitis: a manifestation of systemic lupus erythematosus strongly associated with antiphospholipid antibodies. J Rheumatol. 1990;17(1):34–7.

    PubMed  CAS  Google Scholar 

  56. Kovacs B, Lafferty T, Brent L, DeHoratius R. Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature. Ann Rheum Dis. 2000;59:120–4.

    Article  PubMed  CAS  Google Scholar 

  57. Sherer Y, Hassin S, Shoenfeld Y, et al. Transverse myelitis in patients with antiphospholipid antibodies—the importance of early diagnosis and treatment. Clin Rheumatol. 2002;21:207–10.

    Article  PubMed  CAS  Google Scholar 

  58. Jabs DA, Miller NR, Newman SA, Johnson MA, Stevens MB. Optic neuropathy in systemic lupus erythematosus. Arch Ophthalmol. 1986;104(4):564–8.

    PubMed  CAS  Google Scholar 

  59. Theodoridou A, Settas L. Demyelination in rheumatic diseases. J Neurol Neurosurg Psychiatry. 2006;77:290–5.

    PubMed  CAS  Google Scholar 

  60. Gibbs AN, Moroney J, Foley-Nolan D, O’Connell PG. Neuromyelitis optica (Devic’s syndrome) in systemic lupus erythematosus: a case report. Rheumatology (Oxford). 2002;41(4):470–1.

    Article  CAS  Google Scholar 

  61. Birnbaum J, Kerr D. Devic’s syndrome in a woman with systemic lupus erythematosus: diagnostic and therapeutic implications of testing for the neuromyelitis optica IgG autoantibody. Arthritis Care Res. 2007;57:347–51.

    Article  Google Scholar 

  62. Mathews MK. Nonarteritic anterior ischemic optic neuropathy. Curr Opin Ophthalmol. 2005;16(6):341–5.

    Article  PubMed  Google Scholar 

  63. Birnbaum J, Kerr D. Optic neuritis and recurrent myelitis in a woman with systemic lupus erythematosus. Nat Clin Pract Rheumatol. 2008;4(7):381–6. Epub 2008 May 20.

    PubMed  Google Scholar 

  64. Deodhar AA, Hochenedel T, Bennett RM. Longitudinal involvement of the spinal cord in a patient with lupus related transverse myelitis. J Rheumatol. 1999;26:446–9.

    PubMed  CAS  Google Scholar 

  65. Tellez-Zenteno JF, Remes-Troche JM, Negrete-Pulido RO, et al. Longitudinal myelitis associated with systemic lupus erythematosus: clinical features and magnetic resonance imaging of six cases. Lupus. 2001;10:851–6.

    Article  PubMed  CAS  Google Scholar 

  66. D’Cruz DP, Mellor-Pita S, Joven B, Sanna G, Allanson J, Taylor J, et al. Transverse myelitis as the first manifestation of systemic lupus erythematosus or lupus-like disease: good functional outcome and relevance of antiphospholipid antibodies. J Rheumatol. 2004;31:280–5.

    PubMed  Google Scholar 

  67. de Seze J, Stojkovic T, Ferriby D, et al. Devic’s neuromyelitis optica: clinical, laboratory, MRI and outcome profile. J Neurol Sci. 2002;197(1–2):57–61.

    Article  PubMed  Google Scholar 

  68. •• Min JH, Kim SH, Park MS, et al. Brain MRI lesions characteristic of neuromyelitis optica and positive anti-aquaporin 4-antibody may predict longitudinal extensive myelitis and optic neuritis in Sjögren’s syndrome. Mult Scler. 2010;16(6):762–4. A patient with Sjögren’s syndrome had characteristic brain lesions of NMO with high titers of AQP4 antibodies before developing LETM and ON. This case suggests that brain lesions of NMO associated with systemic autoimmune disorders (especially Sjögren’s syndrome) have to be included in the spectrum of NMO-related disorders.

    Article  PubMed  Google Scholar 

  69. • Squatrito D, Colagrande S, Emmi L. Devic’s syndrome and primary APS: a new immunological overlap. Lupus 2010;19:1337–9. This case report describes a patient meeting the classification criteria for primary APS and the new diagnostic criteria for NMO. It is important to diagnose NMO syndrome, as both ON and transverse myelitis were also considered neurological complications of APS. NMO-IgG is a new and fundamental test to determine whether immunosuppressant therapy is recommended for such patients.

    Article  PubMed  CAS  Google Scholar 

  70. • Mottaghi P, Ashtari F, Karimzadeh H, Seidbonakdar Z, Karimifar M, Salesi M. Devic’s syndrome concomitant with nephritis in a young woman. Clin Rheumatol. 2009;28(10):1239–40.Epub 2009 Aug 1. The authors presented the case of a woman with blurred vision and the inability to walk due to acute ON, transverse myelitis, and NMO, an interesting presentation of this syndrome with proteinuria as the first manifestation of SLE.

    Article  PubMed  Google Scholar 

  71. Protti A, Erminio C, Piccolo I, Spreafico C, Colombo F, Ghezzi A. An unusual case with relapsing neuromyelitis optica associated with undifferentiated connective tissue disease. Neurol Sci. 2004;25 Suppl 4:S383–5.

    Article  PubMed  Google Scholar 

  72. April RS, Vansonnenberg E. A case of neuromyelitis optica (Devic’s syndrome) in systemic lupus erythematosus. Clinicopathologic report and review of the literature. Neurology. 1976;26:1066–70.

    PubMed  CAS  Google Scholar 

  73. Jacobi C, Stingele K, Kretz R, Hartmann M, Storch-Hagenlocher B, Breitbart A, et al. Neuromyelitis optica (Devic’s syndrome) as first manifestation of systemic lupus erythematosus. Lupus. 2006;15:107–9.

    Article  PubMed  CAS  Google Scholar 

  74. Ferreira S, Marques P, Carneiro E, D’Cruz D, Gama G. Devic’s syndrome in systemic lupus erythematosus and probable antiphospholipid syndrome. Rheumatology. 2005;44:693–5.

    Article  PubMed  CAS  Google Scholar 

  75. Hagiwara N, Toyoda K, Uwatoko T, Yasumori K, Ibayashi S, Okada Y. Successful high dose glucocorticoid treatment for subacute neuromyelitis optica with systemic lupus erythematosus. Intern Med. 2005;44:998–1001.

    Article  PubMed  Google Scholar 

  76. Graber DJ, Levy M, Kerr D, Wade WF. Neuromyelitis optica pathogenesis and aquaporin 4. J Neuroinflammation. 2008;5:22.

    Article  PubMed  Google Scholar 

  77. Mehta LR, Samuelsson MK, Schwid SR, et al. Neuromyelitis optica spectrum disorder in a patient with systemic lupus erythematosus and anti-phospholipid antibody syndrome. Mult Scler. 2008;14:425–8.

    Article  PubMed  Google Scholar 

  78. Weinshenker BG. Neuromyelitis optica is distinct from multiple sclerosis. Arch Neurol. 2007;64(6):899–901.

    Article  PubMed  Google Scholar 

  79. Weinshenker BG, Wingerchuk DM, Vukusic S, et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol. 2006;59(3):566–9.

    Article  PubMed  CAS  Google Scholar 

  80. Ferreira S, D’Cruz DP, Hughes GR. Multiple sclerosis, neuropsychiatric lupus and antiphospholipid syndrome: where do we stand? Rheumatology (Oxford). 2005;44(4):434–42. Epub 2005 Jan 11.

    Article  CAS  Google Scholar 

  81. Karussis D, Leker RR, Ashkenazi A, Abramsky O. A subgroup of multiple sclerosis patients with anticardiolipin antibodies and unusual clinical manifestations: do they represent a new nosological entity? Ann Neurol. 1998;44:629–34.

    Article  PubMed  CAS  Google Scholar 

  82. •• Katzav A, Shoenfeld Y, Chapman J. The pathogenesis of neural injury in animal models of the antiphospholipid syndrome. Clin Rev Allergy Immunol. 2010;38:196–200. In this article, the authors propose a logical pathway for how experimental APS causes brain dysfunction. The diffuse brain endothelial dysfunction would result in extravasations of serum proteins, including APS-IgG and activated thrombin, which may induce the behavioral changes observed in the APS mice. They collected data from the mouse experimental APS model that support this hypothesis.

    Article  PubMed  CAS  Google Scholar 

  83. Wingerchuk DM, Lennon VA, Lucchinetti CF, Pittock SJ, Weinshenker BG. The spectrum of neuromyelitis optica. Lancet Neurol. 2007;6(9):805–15.

    Article  PubMed  CAS  Google Scholar 

  84. Wingerchuk DM, Weinshenker BG. Neuromyelitis optica. Curr Treat Options Neurol. 2005;7(3):173–82.

    Article  PubMed  Google Scholar 

  85. Watanabe S, Nakashima I, Misu T, et al. Therapeutic efficacy of plasma exchange in NMO-IgG-positive patients with neuromyelitis optica. Mult Scler. 2007;13(1):128–32.

    Article  PubMed  CAS  Google Scholar 

  86. Keegan M, Pineda AA, McClelland RL, Darby CH, Rodriguez M, Weinshenker BG. Plasma exchange for severe attacks of CNS demyelination: predictors of response. Neurology. 2002;58(1):143–6.

    PubMed  CAS  Google Scholar 

  87. Mandler RN, Ahmed W, Dencoff JE. Devic’s neuromyelitis optica: a prospective study of seven patients treated with prednisone and azathioprine. Neurology. 1998;51:1219–20.

    PubMed  CAS  Google Scholar 

  88. Falcini F, Trapani S, Ricci L, Resti M, Simonini G, de Martino M. Sustained improvement of a girl affected with Devic’s disease over 2 years of mycophenolate mofetil treatment. Rheumatology (Oxford). 2006;45(7):913–5. Epub 2006 Apr 25.

    Article  CAS  Google Scholar 

  89. •• Jacob A, Matiello M, Weinshenker BG, et al. Treatment of neuromyelitis optica with mycophenolate mofetil: retrospective analysis of 24 patients. Arch Neurol. 2009;66(9):1128–33. In this study, the efficacy and safety of mycophenolate mofetil therapy in 24 patients with NMOSDs were evaluated. The authors concluded that mycophenolate is associated with reduction in relapse frequency and stable or reduced disability in patients with NMOSDs.

    Article  PubMed  Google Scholar 

  90. Minagar A, Sheremata WA. Treatment of Devic’s disease with methotrexate and prednisone. Int J MS Care. 2000;2:39–43.

    Google Scholar 

  91. Mok CC, To CH, Mak A, Poon WL. Immunoablative cyclophosphamide for refractory lupus-related neuromyelitis optica. J Rheumatol. 2008;35(1):172–4.

    PubMed  Google Scholar 

  92. • Nasir S, Kerr DA, Birnbaum J. Nineteen episodes of recurrent myelitis in a woman with neuromyelitis optica and systemic lupus erythematosus. Arch Neurol. 2009;66(9):1160–3. Ongoing B-cell stimulation may have led to activation of the cellular arm of the immune system during earlier attacks and contributed to treatment intractability to rituximab during later attacks.

    Article  PubMed  Google Scholar 

  93. Weinstock-Guttman B, Ramanathan M, Lincoff N, et al. Study of mitoxantrone for the treatment of recurrent neuromyelitis optica (Devic disease). Arch Neurol. 2006;63(7):957–63.

    Article  PubMed  Google Scholar 

  94. Fidler JM, DeJoy SQ, Smith III FR, Gibbons Jr JJ. Selective immunomodulation by the antineoplastic agent mitoxantrone, II: nonspecific adherent suppressor cells derived from mitoxantrone-treated mice. J Immunol. 1986;136:2747–54.

    PubMed  CAS  Google Scholar 

  95. Jacob A, Weinshenker B, Violich I, et al. Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients. Arch Neurol. 2008;65:1443–8.

    Article  PubMed  Google Scholar 

  96. •• Kim HJ, Kim W, Park MS, et al. B cell depletion therapy using rituximab in neuromyelitis optica spectrum disorders. Mult Scler 2009;15:S 253. Twenty-seven patients with NMO treated prospectively received multiple infusions of rituximab for up to 2 years to assess the safety and efficacy of this agent.

    Article  Google Scholar 

Download references

Acknowledgment

The authors gratefully thank Raúl Galoppe, PhD, of Montclair State University for his help in translating the manuscript.

Disclosure

No potential conflicts of interest relevant to this article were reported.

Author information

Authors and Affiliations

  1. Rheumatology Section, Hospital Cullen, Alberdi 5699, 3000, Santa Fé, Argentina

    Emilio Benavente

  2. Rheumatology Section, Hospital Cullen, Tucumán 3080, 3000, Santa Fé, Argentina

    Sergio Paira

Authors
  1. Emilio Benavente
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sergio Paira
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sergio Paira.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Benavente, E., Paira, S. Neuromyelitis Optica-AQP4: An Update. Curr Rheumatol Rep 13, 496–505 (2011). https://doi.org/10.1007/s11926-011-0211-9

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11926-011-0211-9

Keywords

Search

Navigation